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ALK 음성 원발성 위 역형성 큰세포 림프종 1예

A Case of Anaplastic Lymphoma Kinase-Negative Primary Gastric Anaplastic Large Cell Lymphoma

  • Kim, Hyeong Seok (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Park, Kun Woo (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Lee, Jung Lim (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Bae, Soo Hyun (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Do, Won Seok (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Kim, Mi Young (Department of Internal Medicine, Daegu Fatima Hospital) ;
  • Lee, Sun Ah (Department of Internal Medicine, Daegu Fatima Hospital)
  • 발행 : 2012.11.01

초록

원발성 림프절외 림프종은 림프절이 아닌 조직이나 기관에서 유래한 림프종으로 위장관에서 가장 호발한다. 역형성 큰세포 림프종은 비호지킨성 림프종의 아형으로 여러 면역 조직화학적 검사가 진단과 예후의 판정에 이용되며 이 중 ALK 단백질은 50-80%에서 발현되는 것으로 양성인 경우에 비해 음성인 경우가 예후가 나쁜 경향을 보인다. 저자들은 고령이며 진행된 병기로 진단된 ALK 음성 원발성 위 역형성 큰세포 림프종 환자에서 복합항암화학요법으로 36개월간 완전관해를 유지하고 있는 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Primary malignant lymphoma of the stomach comprises 1 to 7% of all gastric malignancies and is mostly B-cell type. Anaplastic large cell lymphoma (ALCL) of the stomach is very rare and regarded to have a poor prognosis. A 66-year-old man complained of dyspepsia for 3 days. A esophagogastroduodenoscopic examination revealed a flat, depressed ulcer at the distal antrum. Biopsy of the lesion showed diffuse infiltration of large pleomorphic lymphoid cells that were positive for CD3 and CD30 and negative for cytokeratin and anaplastic lymphoma kinase (ALK). We diagnosed the patient with ALK-negative primary gastric ALCL with multiple lymphadenopathies, and he received systemic chemotherapy. CHOP-like regimens followed by consolidation therapy have been widely used for ALK-negative ALCL. However, the patient maintained complete remission for 36 months with only induction chemotherapy. This case suggests that heavy treatment for ALK-negative ALCL is not required for all patients.

키워드

참고문헌

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